Ataxin-3

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  Subject Areas on Research

  • Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117. 
  • Interaction of the polyglutamine protein ataxin-3 with Rad23 regulates toxicity in Drosophila models of Spinocerebellar Ataxia Type 3. 
  • Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription. 
  • The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains. 
  • The ubiquitin-conjugating enzyme (E2) Ube2w ubiquitinates the N terminus of substrates. 
  • Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP. 
  • Ubiquitin-binding site 2 of ataxin-3 prevents its proteasomal degradation by interacting with Rad23. 
  • Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3.